Scleroderma
Scleroderma is an autoimmune disease characterized by inflammation and the thickening of skin and internal tissues due to an overproduction of collagen.
What is Scleroderma?
Scleroderma means "hard skin." It is an autoimmune disease that causes inflammation and thickening in the skin and other areas of the body. This inflammation results in areas of tight, hard skin. Scleroderma may affect just one area of your body, or it can affect many systems.
Types of Scleroderma
- Localized scleroderma: Only affects your skin and the muscles and tissues just under your skin.
- Systemic scleroderma (systemic sclerosis): A more serious type that affects many parts of your body and can damage your blood vessels and internal organs, such as your heart, lungs, and kidneys.
Causes and Risk Factors
The exact cause is unknown, but researchers believe several factors play a role:
- Genetics: Certain genes can increase the chance of developing the disease. While not passed directly from parent to child, you are more likely to develop it if a close relative has it.
- Environment: Exposure to viruses or chemicals may trigger the disease.
- Immune system changes: Changes can trigger cells to make too much collagen, causing patches of tight, hard skin.
- Hormones: Hormonal or immune system differences between women and men may play a part.
Anyone can develop scleroderma, but it is more common in women, typically appearing between the ages of 30 and 50. While it affects all races, it can affect African Americans more severely.
Symptoms
- Localized scleroderma: Causes patches of thick, hard skin, either in firm, oval shapes (morphea) or lines of thickened skin on the arms, legs, or forehead (linear scleroderma).
- Systemic scleroderma: Can cause thick, tight skin on fingers, fatigue, Raynaud's phenomenon (narrowing of blood vessels in hands or feet), and damage to internal organs.
Diagnosis and Treatment
There is no single test for scleroderma. Diagnosis involves reviewing symptoms and medical history, a physical exam, blood tests (including an ANA test), skin biopsy, and imaging tests to check for organ damage.
While there is no cure, treatments help control symptoms and limit damage:
- Medicines: To decrease swelling, manage pain, and prevent complications.
- Physical or occupational therapy: To improve muscle strength and assist with daily living.
- Regular dental care: To manage dry mouth and prevent tooth decay.
Patients often work with a rheumatologist or dermatologist. Self-management includes dressing warmly, quitting smoking, using sunscreen and moisturizers, avoiding harsh soaps or hot water, and staying physically active.